Pediatric cranial osteoblastoma: Technical note of surgical treatment and review of the literature.

Osteoblastoma of the skull is a rare entity, and they account only for 2-4% of all the cases of osteoblastoma. We perform a comprehensive review of the pertinent literature on the subject and we report a case of a 3-year-old girl presenting with a 6-month history of a supraorbital mass and exophthalmos due to an osteoblastoma of the frontal and ethmoid bones involving the orbit and anterior skull base. A 3D printed model of the patient's skull was used for the preoperative planning and reconstruction strategy. Total en-bloc resection of the tumor followed by immediate reconstruction was achieved. No recurrence was detected 3 years after the surgery. Gross total resection is strongly advised with skull osteoblastoma, especially in young age, because of the risk of the recurrence and malignant transformation. 3D printing is proven to be a valuable tool to enhance surgical performance by avoiding complications while achieving total resection with accurate reconstruction. Long-term follow-up is important to detect recurrences and improve the management of these young patients.

Investigation of bioactivity, biocompatibility and thermal behavior of sol-gel silica glass containing a high PEG percentage.

SiO2/PEG organic-inorganic hybrid materials, which contain 60 or 70 weight percentage of PEG, were synthesized by the sol-gel technique. The materials were characterized and subjected to various tests to assess their application in the biomedical field. The evaluation of their morphology by scanning electron microscopy (SEM) confirms the homogeneity of the samples on the nanometer scale. Fourier transform infrared spectroscopy (FT-IR) indicated that the two components of the hybrids (SiO2 and PEG) are linked by hydrogen bonds. This feature makes them class I hybrids. Simultaneous thermogravimetry/differential thermal analysis (TG/DTA) was used to investigate their thermal behavior and to establish the best temperatures for their pre-treatment. The fundamental properties that a material must have to be used in the biomedical field are biocompatibility and bioactivity. The formation of a hydroxyapatite layer was observed on the hybrid surface by SEM/EDX and FTIR after soaking in simulated body fluid. This indicates that the materials are able to bond to bone tissue. Moreover, the biocompatibility of SiO2/PEG hybrids was assessed by performing WST-8 cytotoxicity tests on fibroblast cell NIH 3T3 after 24h of exposure. The cytotoxicity tests highlight that the cell viability is affected by the polymer percentage. The results showed that the synthesized materials were bioactive and biocompatible. Therefore, the results obtained are encouraging for the use of the obtained hybrids in dental or orthopedic applications.

Exploring the association of succinate dehydrogenase complex mutations with lymphoid malignancies.

The succinate dehydrogenase (SDH) complex exerts a fundamental role in mitochondrial cellular respiration and mutations in its encoding genes (SDHA, SDHB, SDHC, SDHD, collectively referred to as SDHx) lead to a number of inherited endocrine cancer predisposition syndromes, including familial paraganglioma/pheochromocytoma. Recent studies suggest a possible role for the SDH complex and other mitochondrial enzymes in the pathogenesis of hematological malignancy. Our aim was to search and identify pedigrees of patients affected by germline SHDx mutations treated at our institution for endocrine and other tumors, and seek to identify cases of hematological malignancy. We also analyzed cancer genome databases for reported cases of SDHx mutations outside of endocrine neoplasms. We report of two unrelated pedigrees carrying SDHx mutations with members affected by lymphomas. Sequencing data revealed one case of chronic lymphocytic leukemia with a SDHB mutation. This novel set of observations demonstrates the need for collaborative databases of patients with endocrine cancers with SDHx mutations, and the investigation of their role in hematological (lymphoid) malignancy.

Can smaller-scale comprehensive cancer centers provide outstanding care in abdominal and thoracic pediatric solid tumor surgery? Results of a 14-year retrospective single-center analysis.

PURPOSE: Quality of care and its measurement represent a considerable challenge for pediatric smaller-scale comprehensive cancer centers (pSSCC) providing surgical oncology services. It remains unclear whether center size and/or yearly case-flow numbers influence the quality of care, and therefore impact outcomes for this population of patients. PATIENTS AND METHODS: We performed a 14-year, retrospective, single-center analysis, assessing adherence to treatment protocols and surgical adverse events as quality indicators in abdominal and thoracic pediatric solid tumor surgery. RESULTS: Forty-eight patients, enrolled in a research-associated treatment protocol, underwent 51 cancer-oriented surgical procedures. All the protocols contain precise technical criteria, indications, and instructions for tumor surgery. Overall, compliance with such items was very high, with 997/1,035 items (95 %) meeting protocol requirements. There was no surgical mortality. Twenty-one patients (43 %) had one or more complications, for a total of 34 complications (66 % of procedures). Overall, 85 % of complications were grade 1 or 2 according to Clavien-Dindo classification requiring observation or minor medical treatment. Case-sample and outcome/effectiveness data were comparable to published series. Overall, our data suggest that even with the modest caseload of a pSSCC within a Swiss tertiary academic hospital, compliance with international standards can be very high, and the incidence of adverse events can be kept minimal. CONCLUSION: Open and objective data sharing, and discussion between pSSCCs, will ultimately benefit our patient populations. Our study is an initial step towards the enhancement of critical self-review and quality-of-care measurements in this setting.

Neuroendoscopic management of a solitary pineal region tumor. Case report of an adenocarcinoma metastasis.

The present case describes a two-step endoscopic management of hydrocephalus and diagnosis of a single pineal region metastasis arising from a gastric adenocarcinoma. A 62-year-old man presenting with signs of subacute obstructive hydrocephalus from a pineal region mass had at first been treated with an endoscopic third ventriculostomy. As cerebrospinal fluid tumor markers (alpha-fetoprotein, beta-human chorionic gonadotropin) were negative, an endoscopic biopsy of the pineal region tumor was performed through a more anterior frontal burr hole. Pathology showed an adenocarcinoma and primary tumor work-up revealed an unsuspected gastric tumor, the pathology of which matched with the intracranial metastasis. The present report emphasizes the role of neuroendoscopy in pineal region tumors and reports a rare case of a solitary gastric adenocarcinoma metastasis in this location.

Increased risk of acute myeloid leukaemia after treatment for breast cancer.

This study evaluates the risk of acute myeloid leukaemia (AML) in patients treated for breast cancer. We included all 6360 breast cancer patients that were recorded at the Geneva Cancer Registry between 1970 and 1999. Patients were followed for AML occurrence until December 2000. We calculated standardized incidence ratios of AML and identified factors modifying the risk of AML by multivariate Cox analysis. Twelve (0.2%) patients developed AML. In general, patients treated for breast cancer had a 3.5-fold (95% confidence interval (CI): 1.8-6.0) increased risk of developing AML compared with the general population. In particular, patients who were older than 70 years at breast cancer diagnosis and those treated with radiotherapy (with or without chemotherapy) had a significantly increased risk of developing AML. This population-based study confirms that radiotherapy increases the risk of AML. Due to the relatively low number of women treated with chemotherapy without radiotherapy and due to the infrequency of the disease, the question of whether chemotherapy alone increases this risk of AML cannot yet be answered.

[Undifferentiated sarcoma of the liver: a rare tumor of childhood]. / Le sarcome indifférencié du foie: une tumeur rare chez l'enfant.

UNLABELLED: Undifferentiated sarcoma of the liver is a rare primary tumor of childhood: only about 150 cases have been reported in the literature. CASE-REPORT: A 10 year-old girl was admitted because of diarrhea and weight loss. Sonography, then CT-scan and MRI showed a large tumor of the liver. COMMENTS: In the differential diagnosis of primary liver tumors in children, one should think about undifferentiated sarcoma of the liver, especially if imaging shows haemorrhagic foci and if sonography and CT/MRI display a discordant appearance. Survival has improved in the last decade due to agressive surgery and intensive chemotherapy.

Management of women with ductal carcinoma in situ of the breast: a population-based study.

BACKGROUND: Increasing incidence of ductal carcinoma in situ (DCIS) confronts patients and clinicians with optimal treatment decisions. This multidisciplinary study investigates therapeutic modalities of DCIS in daily practice and provides recommendations on how to increase quality of care. PATIENTS AND METHODS: All women (n = 116) with unilateral DCIS recorded in the Geneva Cancer Registry from 1995 to 1999 were considered. Information concerned patient and tumor characteristics, treatment and outcome. Factors linked to therapy were determined using a case-control approach. Cases were women with treatment of interest and controls other women on the study. RESULTS: Most DCIS cases (62%) were discovered by mammography screening. Ninety (78%) women had breast-conserving surgery (BCS), 18 (16%) mastectomy and seven (6%) bilateral mastectomy. Eight (7%) patients had tumor-positive margins, 18 (16%) lymph node dissection and two (1.7%) chemotherapy. Twenty-five per cent of women with BCS had no radiotherapy, three had radiotherapy after mastectomy. Less than 50% underwent breast reconstruction after mastectomy. Method of discovery, multifocality, tumor localization, size and differentiation were linked to the use of BCS or lymph node dissection. CONCLUSIONS: Because of important disparities in DCIS management, recommendations are made to increase quality of care, in particular to prevent axillary dissection or bilateral mastectomy and to increase the use of radiotherapy after BCS.

[Evaluation of the impact of guidelines for rationalizing the prescription of preop tests for patients ASA 1 and 2 undergoing elective surgery]. / Valutazione dell'impatto di linee-guida per la razionalizzazione della prescrizione degli esami preoperatori a pazienti ASA 1 e 2 sottoposti a chirurgia elettiva.

OBJECTIVES: To evaluate the impact of implementation of guidelines aimed at reducing the use of laboratory and diagnostic preoperative tests in patients with low anesthesiologic risk, admitted to six public hospital of Ente Ospedaliero Cantonale in Cantone Ticino (Switzerland). DESIGN: Observational study. METHODS: Time series analysis of patterns of use of preoperative tests, on 14,585 patients admitted to public hospitals form March 1996 to June 1998. PRINCIPAL OUTCOME MEASURE: Proportion of patients undergoing a laboratory or other diagnostic tests during three six months periods before guidelines implementation (baseline), during the six months of the implementation phase and during the following four months of adoption of the guidelines in the participating centres. RESULTS: During the four months following the implementation, we observed a reduction of 15% (95% CI: 1%-27%) in the use of azotemia, and a reduction of 34% (95% CI: 18%-50%) for coagulation tests. Corresponding figures for glycemia and chest x-ray indicated a reduction of 44% (95% CI: 32%-54%) and of 22% (95% CI: 8%-34%), respectively. As for other tests (creatinine, ECG), no statistically significant reduction was observed. Most of the observed effect was explained by a reduction in use in patients at a low risk (ASA 1 and 2). In addition, guidelines appeared to have a greater impact in the four small (i.e. < 200 beds) hospitals, as compared with the two centres of greater size. CONCLUSIONS: Consistently with the empirical evidence available in this area, this study suggests that guidelines can change clinical practice, when they are implemented through a strategy taking into account adaptation of the recommendations to local circumstances and involvement of health professionals.

Nitrite and nitrate levels in cerebrospinal fluid of normal subjects.

In order to evaluate the involvement of nitric oxide in neurologic disorders it is important to generate controlled values of its metabolites nitrite and nitrate in human cerebrospinal fluid (CSF). Samples of CSF obtained from 14 patients without neurologic diseases were analysed for nitrite and nitrate concentration by reverse phase chromatography with ultraviolet (UV) detection. For comparison, the levels of nitrite in the same samples were also measured by reverse phase chromatography coupled with electrochemical detection and those of nitrate by ion chromatography coupled with UV detection. A good correlation was found for the concentration values of both ions obtained with the two procedures. Then, 10.41 +/- 0.47 ng/ml of nitrite and 2.92 +/- 0.37 ng/ml of nitrate could be regarded as reliable values in control subjects. No correlation between age and levels of nitrite and nitrate was observed.

[Intraoperative monitoring: visual evoked potentials in surgery of the sellar region]. / Intraoperatives Monitoring: Visuell evozierte Potentiale bei Eingriffen in der Sellaregion.

During 18 sellar and perisellar operations the optic tract was monitored by visual evoked potentials (VEP). Deteriorations of the cortical responses were recorded in 73%. In this patients there was no close correlation between the intraoperative findings and the postoperative visual function. Only in those patients who showed no remarkable intraoperative changes VEP seemed to be of reliable prognostic value.

Microsurgical anatomy of the anterior choroidal artery.

The anterior choroidal artery (A.ch.a.) was studied in 140 hemispheres of the human brain. In 50 specimens the artery was cannulated and perfused with acrylic resin, in 20 specimens the A.ch.a., was selectively cannulated and perfused with dye. The artery then was microsurgically dissected and photographically documented. The site of origin of the A.ch.a., its length, outer diameter, course, branching patterns, anastomoses, and areas of supply was recorded. The surgical and diagnostic significance of the A.ch.a. and its importance during surgical procedures are discussed.

[Anesthesiological care of female patients with cerebrovascular aneurysms in pregnancy]. / Anästhesiologische Betreuung von Patientinnen mit Hirngefässaneurysmen in der Schwangerschaft.

This is a report on a patient who suffered subarachnoid haemorrhage from an aneurysm of the a. cerebri media during the 28th week of pregnancy. The aneurysm was clipped under neuroleptanalgesia. Besides monitoring of heart rate, blood pressure and central venous pressure as well as the treatment parameters including end-expiratory CO2, the foetal heart rate was deduced by Doppler sonography. She was hyperventilated intraoperatively with a paCO2 of 3.8-4.1 kPa. During the actual aneurysm surgery, artificial hypotension by 20 mmHg to b.p. values around 100/160 mmHg was initiated by means of nitroglycerin. It is evident from the literature compiled on this occasion that it is mandatory to monitor foetal heart rate during controlled hypotension in pregnancy. Sodium nitroprusside should not be used to initiate artificial hypotension during pregnancy, since it may result in toxic cyanide levels in the foetus. As usual in neurosurgery, hyperventilation can be performed also during pregnancy, since placental blood flow decreases only if the CO2 concentration drops to a very low level.

Microsurgical approach to the cerebellomedullar region in rat.

A microsurgical study was carried out on 20 randomized rats in order to delineate a safe technique for cerebellobulbar approaches. The procedure takes 20-30 min and can be performed with minimum blood loss if anatomical landmarks, particularly the m. obliquus capitis cranialis, are respected.

[Fracture of the occipital condyle. Case description and review of the literature]. / Fraktur des Condylus occipitalis. Fallbeschreibung und Literaturübersicht.

The case of an 18 year old young man with an occipital condyle fracture following a motorcycle accident is reported. Guided by this observation and 10 additional case reports of the literature the mechanism of injury, symptoms and diagnostic procedures of this very rare fracture are discussed in detail.